Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000471724 | SCV000549128 | pathogenic | Danon disease | 2023-12-23 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Leu325Profs*25) in the LAMP2 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in LAMP2 are known to be pathogenic (PMID: 21415759). This variant is not present in population databases (gnomAD no frequency). This premature translational stop signal has been observed in individual(s) with Danon disease (PMID: 27179547). This variant is also known as c.973insC. ClinVar contains an entry for this variant (Variation ID: 409224). For these reasons, this variant has been classified as Pathogenic. |